T lymphocyte colony formation (T-CFU) of patients with non-Hodgkin's lymphoma (mainly aleukemic) were examined by methylcellulose one-step method. T-CFU of untreated patients were 41.2±61.2/5×10⁴ cells compared with 192.0±94.3/5×10⁴ cells of normal subjects. T-CFU of B cell type lymphoma were markedly depressed (22.0±26.0/5×10⁴ cells), whereas T-CFU of T cell type lymphoma were nearly normal range (125.3±73.4/5×10⁴ cells). After achievement of complete remission by chemotherapy, T-CFU tends to recover. On the other hand, after radiation therapy T-CFU were markedly depressed and the depression continued over 6 months.

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Hairy cell leukemia (HCL) in the Japanese patients is not only rare but is atypical in certain clinical and hematological features. In order to investigate if Japanese HCL is atypical also in the immunophenotype, 11 patients with HCL and 11 control patients with non-Hodgkin's lymphomas were studied, utilizing their frozen mononuclear cells which had been stored for up to 5.5 years.
After thawing and trypane blue exclusion, the viable cells were partly tested for M-rosetting and mostly processed for light- and electron-microscopic examinations after fixation with PLP fixative, frozen section and immuno-staining using a panel of monoclonal antibodies.
Results indicated Japanese HCL to derive from a late stage mature B cells because most patients were phenotyped as Smlg⁺, HLA-DR⁺, B1⁺, B2^-, FMC7⁺, Tac⁺, just as would be the case for non-Japanese patients. Remaining phenotypes, however, were J5⁺, Leu1⁺. αHC1^-, αHC2^- in contrast to J5^-, Leu1^-, αHC1⁺, αHC2⁺ known for most non-Japanese patients. We conclude Japanese HCL constitutes a special variant of HCL because it differs from the rest in clinical, hematologic, and immunologic features.

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The results of deoxyuridine suppression test on 142 patients with a wide range of hematological disorders including 46 megaloblastic anemia as obtained by our simplified method are summarized. Major modifications included: 1) simultaneous addition of deoxyuridine (UdR) and ³H-thymidine, 2) 60 min incubation without preincubation with UdR, 3) use of 20% dialyzed calf serum in place of autologous serum, 4) a lower concentration of added UdR (44μM vs 100∼250μM).
UdR suppression values in untreated megaloblastic anemia ranged 19.5∼66.2% with a mean of 48.9%, while it was always less than 10% with a mean and SD of 5.0±1.5% in other conditions including patients with erythroleukemia and myelodysplastic syndromes showing megaloblastic alterations. Thus our modified method may be used as a useful diagnostic aid with sufficient specificity for megaloblastic anemia.

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We have investigated the expression of common acute lymphoblastic leukemia antigen (CALLA), P24 and P20 antigens on non-T, non-B acute lymphoblastic leukemia (ALL) cells and non-hematopoietic tissues, using the murine monoclonal antibodies (SJ-51B4, SJ-9A4 and SJ-43B11), Out of 91 cases with non-T, non-B ALL, CALLA, P24 antigen and P20 antigen were positive in 71 cases, 75 cases and 75 cases, respectively. Although morphologically identical, non-T, non-B ALL cells could be subdivided into phenotypically-defined subgroups on the basis of CALLA, P24 and P20 antigens.
We also analyzed the expression of CALLA, P24 antigen and P20 antigen in nonhematopoietic tissues. CALLA was detected in renal proximal tubule cells and the glomerular basement membrane of the kidney. P24 antigen was widely expressed by nonhematopoietic tissues, e. g. renal distal tubule cells, parietal cells and endothelial cells in the renal glomeruli, capillary endothelium, smooth muscle, alveolar epithelium and stromal cells of the testis. P20 antigen was not reactive with any non-hematopoietic tissues tested in this study.
These findings suggested that the expression of CALLA and P24 antigen is not limited to the cells of lymphohemopoietic lineage. Further study should be undertaken to clarify the biological functions of these leukemia-associated antigens.

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The efficacy of AVC regimen on childhood acute non-lymphocytic leukemia was evaluated. Induction chemotherapy consisted of Ara-C (160mg/m²) given in a continuous IV infusion over 7 consecutive days, combined with adriamycin (50mg/m²) and vincristine (1mg/m²). After 1 course of consolidation therapy intensification therapies were repeated every 2 months for 3 years and every 3 months for 1 year. Intensification therapies were modified AVC regimen for the first 2 years and a combination of intermediate dose (500mg/m²) Ara-C (every 12 hours for 4 days) and 2 doses of VP-16-213 (150mg/m²) for 2 years thereafter.
A complete remission was achieved in 4 of 5 patients, after 1 course of induction therapy by 3 patients and after 2 courses by 1 patient. The remission durations of these 4 patients have ranged from 9 to 27 months. None has relapsed. Toxicities of this regimen included bone marrow suppression, vomiting, fever, exnthem and probable infection. However, this regimen seemed to be tolerable for most patients.
Although a longitudinal trial with a large number of patients is needed to draw a definitive conclusion, our study indicated that this regimen could be an excellent therapeutic strategy.

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We investigated the functions of polymorphonuclear leukocytes (PMNs) obtained from patients with liver cirrhosis since these patients have an increased susceptibility to infections. PMNs of the patients with cirrhosis showed phagocytic and bactericidal activities similar to those of normal controls whether the assays were performed in the presence of normal serum or of patients' serum. Chemotaxis-inrducing activity of the patients' serum was significantly reduced compared to that of normal serum. The release of O^-₂ by PMNs of patients in response to phorbol myristate acetate (PMA) was greater than that of normal PMNs. However, O^-₂ release in response to opsonized zymosan (OZ) or without stimulant and ·OH generation were almost the same with patients' PMNs and normal PMNs. These data suggest that the decrease of chemotaxis-inducing activity of the patients' serum might cause the defect for PMNs to accumulate in the inflammatory focus, and this might be one of the reasons for increasing susceptibility to bacterial infections.

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Results of treatment were analysed in 25 patients with Hodgkin's disease who were admitted to the Division of Hematology, Jichi Medical School Hospital between 1974 and 1985. The median age of the patients was 48 years. According to the Rye classification, the patients were divided as follows; 9 nodular sclerosis, 6 mixed cellularity, 6 lymphocytic depletion and 4 lymphocytic predominance. Twenty-one out of 25 patients were in advanced stages; 10 in stage III and 11 stage IV, and 20 patients were positive for B symptom.
All patients except one received COPP or VEMP chemotherapy. The median survival of them was 55 months and the actuarial survival at 5 years and 10 years were 41% and 25%, respectively. The patients with mixed cellularity had a worse prognosis than those with nodular sclerosis or lymphocytic predominance. The patients with stage IV disease also had a poor prognosis.
Sometimes it is difficult to differentiate Hodgkin's disease from some types of the non-Hodgkin's lymphoma such as pleomorphic T-cell lymphoma on pathological findings. Misdiagnosis of these lymphomas might be one of the reason for the poor prognosis of Hodgkin's disease in Japan as well as in our series. To improve the prognosis of patients with Hodgkin's disease, correct diagnosis and staging would be neccessary.

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Results of treatment were analysed in 84 patients with non-Hodgkin's lymphoma who were admitted to the Division of Hematology, Jichi Medical School Hospital between 1974 and 1985. The median age of the patients was 56.5 years. Majority of the patients (83.3%) were of nodal origin, while 7.1% of Waldeyer's ring and only 9.6% of extranodal origin. According to the LSG classification, the patients who entered after 1979 were divided as follows; 37.7% diffuse large, 16.4% diffuse medium, 9.8% diffuse mixed, 6.6% either diffuse lymphoblastic, diffuse pleomorphic or diffuse small, while only 8.2% of the patients had follicular subtypes. Distribution by clinical stage was; stage I 2 (2.4%), II 10 (11.9%), III 25 (29.8%), IV 47 (56.0%).
The following combinations were used mainly as induction therapy between 1974 and 1983; COPP (cyclophosphamide, vincristine, procarbazine and prednisolone), VEMP (vincristine, cyclophosphamide, 6-merucaptopurine and prednisolone), CHOP (cyclophosphamide, adriamycin, vincristine and prednisolone) and VEPA (vindesine, cyclophosphamide, prednisolone and adriamycin).
The overall response rate of the 50 patients treated with above regimens was 52% (CR 28%, PR 24%). Response rates were significantly higher with CHOP/VEPA regimens (76%) than with other regimens (19%) (p<0.001). The median survival of these patients was 18 months and the five-year survival rate was 24%. The patients treated with CHOP/VEPA survived significantly longer than those with other regimens, the median survival was 36 months and the 5-year survival rate was 38% in the former. The patients who achieved a complete remission early also had a favorable prognosis with the median survival of 91 months and the 5-year survival rate of 64%.
There was no statistical differences in the actuarial survival as well as the responce rates according to the histological subtypes. However, the prognosis closely related to the clinical stage; the median survival and the 5-year survival rate were 43 months and 50%, respectively in stage III patients, while 12 months and 7.2% in stage IV patients (p=0.002).
Since 1984, intensified CHOP with high-dose methotrexate (M-CHOP) was given as initial treatment for patients with advanced “bad-risk” lymphoma. Of 15 patients, 5 achieved complete remission and 9 achieved partial remission. The median follow up is 17 months and only 1 death has been recorded in these patients. These findings demonstrate that intensive treatment is necessary for long-term remission and cure for the patients with bad-risk non-Hodgikin's lymphoma.

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Twenty six patients with acute nonlymphocytic leukemia (ANLL), 12 patients with myelodysplastic syndromes (MDS), none of whom received intensive chemotherapy, and 17 patients with relapsed and/or refractory ANLL were treated with low dose Ara-C therapy (LDAC) (10mg/m²/12hr S.G.). In cases who received no intensive chemotherapy, 15 patients with ANLL and 2 patients with MDS obtained complete remission (CR) and a patient with ANLL and 6 patients with MDS obtained partial remission. In refractory and/or relapsed ANLL cases only 4 patients obtained CR and a patient obtained PR. Patients with M1 and M2 achieved high response rates compared with M3, M5 and M6. In ANLL who received no intensive chemotherapy there was no apparent correlation between response and bone marrow cellularity, percentage or number of blasts in bone marrow. Duration of responses with ANLL without previous intensive chemotherapy, relapsed and/or refractory ANLL and MDS were from 3M to 17M (median 8M), from 13M to 42M (median 23M) and from 2M to 12M (median 6M) respectively. The median survival for all cases and responders was 16M and 22M in ANLL without previous intensive chemotherapy. 4M, 23M in relapsed and/or refractory ANLL and 31M and 31M in MDS. Present results show that treatment with low dose Ara-C may be effective in fresh ANLL patients with advanced age, hypoplastic bone marrow and/or peripheral cytopenia or severe complications. However further investigation is necessary to evaluate whether the therapy prolong the survival in MDS.

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Between December 8, 1984 and February 8, 1985, 47 patients with hematologic malignancies were studied for the relation between the serum D-arabinitol values and neutrophil counts. The incidence (13 cases, 87%) of neutropenia among 15 patients with high serum D-arabinitol concentrations and high D-arabinitol/creatinine ratios was high. One patient without neutropenia was diagnosed as adult T-cell leukemia. Five patients with neutropenia, fever refractory antibiotics and/or undergoing intensive induction chemotherapy were given antifungal agents. In 7 cases, when neutrophil counts increased, the D-arabinitol concentrations and creatinine ratios turned to normal range. Only 2 cases of 15 patients with neutropenia (less than 500/μl) showed normal D-arabinitol levels.
These results suggest that the determination of serum D-arabinitol concentration and its creatinine ratio may be helpful in the diagnosis of invasive candidiasis prior to the antifungal therapy, especially when the patient is treated with intensive induction chemotherapy or has severe neutropenia.

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We have experienced that marked reduction of the hemoglobin arised frequently after combination chemotherapy with N⁴-behenoyl-1-β-D-arabinofuranosylcytosine (BHAC) in acute leukemia. Because BHAC was suspected as a cause of the hemoglobin reduction, its influence on human erythrocytes was investigated.
Erythrocyte suspensions were prepared from blood of healthy donors and cultured with BHAC in a CO₂ incubator. Osmotic fragility of erythrocytes was determined along with the incubation up to 24 hours. The effect of Ara-C and the solvent of BHAC (HCO-60) was also examined. BHAC increased osmotic fragility of erythrocytes which was dependent on the duration of incubation time and on the concentrations. HCO-60 also caused the increase of osmotic fragility of erythrocytes, but its extent was far small compared with that of BHAC. Ara-C didn't have significant effect on osmotic fragility of erythrocytes. It was concluded that such a effect of BHAC and HCO-60 on human erythrocytes might explain the reduction of the hemoglobin that was observed frequently when BHAC was used for the treatment of leukemia.

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Cyclosporine was given to 14 children who received allogeneic bone marrow transplants as therapy for aplastic anemia and hematologic malignancies. Cyclosporine alone was administered to 6 patients. Other 8 patients received the combination of methotrexate and cyclosporine for the prophylaxis of graft-versus-host disease (GVHD). Eleven patients had HLA identical sibling donors. Other 3 patients were grafted from HLA mismatched family donors.
Acute GVHD developed in 4 patients, of whom one had grade I and three grade II. Five of the 10 patients who were alive more than 4 months after marrow grafting developed chronic GVHD. Renal functional abnormality was seen in 5 patients. Trough levels of serum cyclosporine were well correlated with the presence of renal dysfunction. Five patients developed hypertension. Methylpredonisolone pulse therapy contributed significantly to the hypertention. One patient died from recurrence of the disease and the other from acute renal failure probably due to cyclosporine. Twelve patients are alive from 2 to 37 months after transplants with a median follow up of 8 months.

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We report a case of refractory anemia (RA) associated with pyoderma gangrenosum (PG). A 31-year old man who suffered from PG was admitted to our hospital for the further evaluation of pancytopenia.
The diagnosis of RA was made from the following findings: chronic irreversible course of pancytopenia, absence of underlying diseases or drugs cellular marrow, morphological abnormalities in all three cell series, increased blasts (4%) in bone marrow but no blasts in peripheral blood.
He had immunological abnormalities such as decreased activity of O^-₂ generation and phagocytosis of neutrophils.
Reviewing literatures, we found 38 cases of various forms of leukemia associated with PG heretofore reported.

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A 10-year old female of hyperthyroidism developed aplastic anemia during methimazole therapy. In order to determine the mechanisms of pancytopenia, we examined the effects of patient's serum, drug and or peripheral blood mononuclear cells (MNC) on hemopoietic progenitor growth.
Numbers of hemopoietic progenitors including CFU-Mix from patient's bone marrow were significantly reduced. Colony formation of various hemopoietic progenitors from allogeneic and autologous recovered marrow was significantly inhibited by the addition of patient's acute serum. Patient's recovered serum failed to inhibit the colony growth of both bone marrow cells. These findings suggest an immunological mechanism for pancytopenia in our patient.

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A 65-year-old man with diabetes mellitus was refered to our hospital for evaluation of his leukocytosis. Hepatosplenomegaly was observed and laboratory data disclosed leukocytosis with a moderately increased number in immature granulocytes and monocytes in the peripheral blood. These monocytes had a phagocytic activity and were weakly positive for peroxidase staining, but clearly positive for naphthol AS-D chloroacetate esterase staining. A bone marrow aspiration proved to be hypercellular with 11.6% promyelocytes, 9.2% monocytes and 4 4% promonocytes. Cytogenetic examination of circulating mononuclear cells revealed a 45XY, -7, t (2P-, 11q-) karyotype. From these findings, the diagnosis of chronic myelomonocytic leukemia (CMMoL) could be made.
After admission, he was treated twice with low dose Ara-C therapy ineffectively. When white blood cells gradually increased in numbers, other antileukemic agents were transiently effective. At that time, however, dyspnea developed in comparison with the degree of leukocytosis. When WBC rapidly elevated again in spite of the treatment. the patient suffered from severe pulmonary insufficiency and died. An autopsy showed widespread leukemic infiltration of the bone marrow, spleen, liver and in particular, the alveolar walls of the lung.
Leukemic infiltration is frequently observed in the lung of autopsied cases. However, this case seems to be rare, since the leukemic infiltration of the lung may have been the cause of death.

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The hematological effect of recombinant Interferon-alpha (r-IFN-α) was studied on two cases of chronic myelogenous leukemia (CML) with positive Philadelphia chromosome (Ph¹), which became resistant to busulfan. r-IFN-α was given daily at the dose of 1,000×10⁴ units as induction dose. Any adverse effect was not observed. The numbers of white cells and platelets gradually decreased to normal and the number of nucleated cells of bone marrow also decreased to 11.0% and 3.4% of the pretreatment levels, respectively within two months. In one patient, chromosome study revealed the appearance of Ph¹-negative clone and both patients continued hematological remission state at the maintenance dose of 300×10⁴ units, for about one year. It is suggested that r-IFN-α may delay the progress of CML to blastic transformation, even it is given in the accelerated phase.

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A 26-year-old male, born in Hokkaido, Japan, was admitted to the hospital because of bilateral diffuse interstitial shadows in the chest radiogram. Atypical lymphocytes were found in the peripheral blood and in the bronchoalveolar lavage fluid (BALF). Antibody against ATL-associated antigen (ATLA) was positive in his serum. ATLA was expressed in the lymphocytes. Human T-cell leukemia/lymphoma virus type 1 (HTLV-1) proviral DNA sequence was detected in the cellular DNA. On the other hand, the serum antibody titers to Epstein-Barr virus (EBV) were at high level. Phenotypic analysis of the leukemic cells was performed with surface markers by monoclonal antibodies. Peripheral blood lymphocytes were composed of OKT3⁺: 87.4%, OKT4⁺: 45.6%, OKT8⁺: 39.7%. BALF lymphocytes were composed of OKT3⁺: 99.0%, OKT4⁺: 38.0%, OKT8⁺: 69.5%. By means of two-color fluorescence flow cytometory, the peripheral blood lymphocytes separated the OKT4⁺/8^- cell population from the OKT4^-/8⁺ cell population. Almost no OKT4⁺/8⁺ cells were detected. Concerning the size of the cells, both the OKT4⁺ and the OKT8⁺ lymphocytes had nearly the same number of abnormal large lymphocytes, and morphologically, both populations had abnormally shaped lymphocytes.
These results suggest that the leukemic cells of the reported case were composed of two populations of T-lymphocytes that had helper/inducer markers or suppressor/cytotoxic markers.

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We report an adult patient with acute lymphoblastic leukemia (ALL) who developed progressive CNS involvement in the cerebellum, the spine and the frontal lobes after 8 years of complete remission.
A 37-year-old male was admitted to Keio University Hospital because of muscle weakness of both legs in Nov. 1985. He had been diagnosed as ALL at the age of 23 in 1973, being then in complete remission for 8 years, until he was readmitted because of neurological abnormalities caused by the cerebellar mass in 1981. With tumorectomy and cranial irradiation the mass lesion disappeared.
On admission bone marrow was proved to be still in remission, however, many leukemic cells were documented in the cerebrospinal fluid and the myelography revealed a blockade at the lumbar spine. In spite of the intensive treatment including spinal irradiation, laminectomy and intraventricular chemotherapy through an Ommaya reservoir, paraplegia was not improved. Furthermore, an enhanced CT scan showed multiple mass lesions in the frontal lobes. The specimen of the cauda equina at laminectomy showed diffuse infiltration of lymphoblasts, which were demonstrated to be identical to those initially diagnosed as well as those of the recurrence in the cerebellum. Since admission bone marrow has remained in remission, and he is still alive.
Reviewing literatures, we found that CNS in very rare as the primary site of relapse after more than 5 years initial complete remission in adult patients with acute leukemia. In addition, poor prognosis is suggested because of sequential bone marrow relapse. In the present case, however, bone marrow has remained in remission for more than 6 years since the cerebellar involvement.

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A 23-year-old man complaining of palpitations and black outs was admitted to our hospital in September 1985.
Laboratory examinations of peripheral blood revealed RBC 163×10⁴/μl, Hb 6.2 g/dl, Ht 19.0%, reticulocytes 7,570/μl, platelets 1.8×10⁴/μl, and WBC 1,700/μl with 20% granulocytes. Bone marrow aspiration showed NCC 2.0×10⁴/μl, megakaryocytes less than 6.25/μl and abnormal cells. Blood chemistry showed serum iron 211 μg/dl, total iron binding capacity 215 μg/dl and normal value of vitamine B₁₂ and folic acid. Because of pancytopenia and bone marrow hypoplasia, we diagnosed severe aplastic anemia.
Lacking an indentical HLA-type donor for a bone marrow transplantation, we treated the patient with bolus methylprednisolone at a dosage of 20mg/kg administered intravenously begining September 21 1985. Since this therapy did not improve the patient's hematological features, on November 25, 1985 we begun administering antithymocyte globulin (ATG, made in China) at dosage of 20mg/kg intravenously for four days. Ten weeks after ATG therapy, the blood cell counts of the patient revealed WBC 1,500/μl, granulocytes 600/μl, reticulocytes 42,808/μl and platelets 5.8×10⁴/μl and bone marrow aspiration showed NCC 11.5×10⁴/μl. In addition, 12 months after ATG therapy hematological examinations revealed Hb 15.7g/dl, a granulocyte count of 1,581/μl and a platelet count of 9.2×10⁴/μl. Treatment using a low dowsage of ATG showed marked improvement in this case. Therefore, ATG therapy should be tested and studied in other cases of severe aplastic anemia when bone marrow transplantation cannot be performed. ATG therapy may replace bone marrow transplantation as successful treatment in many patients.

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Case 1: A 49 year-old female was diagnosed as pure red cell aplasia (PRCA) in June, 1983. Laboratory findings on admission revealed a Hb of 8.5g/dl and reticulocyte count of 0.2%. The ratio of OKT4/8 was 0.63. The bone marrow revealed erythroid hypoplasia (0.2%). In vitro erythroid colony assay in methyl-cellulose showed a decreased growth of BFU-E (29.3±2.9/10⁵ bone marrow mononuclear cells, BM-MNC) and CFU-E (43.3±3.5/10⁵ BM-MNC). Successive treatments with prednisolone, cyclophosphamide and danazol were unsuccessful. A trial of lymphocytapheresis also failed to induce a reticulocytosis. Seven days after administration of anti-lymphocyte globulin (ALG), the patient achieved a remission for only half a month.
In September, 1986, oral administration of cyclosporine (CsA), 200mg daily, was started. From day 30 of CsA treatment, an increase of reticulocyte count was noticeable.
Case 2: A 52 year-old female was diagnosed as PRCA with thymoma in November, 1985. Laboratory findings on admission revealed a Hb of 8.0g/dl and reticulocyte count of 0.0%. The ratio of OKT 4/8 was 0.58. The bone marrow revealed erythroid aplasia. The growth of bone marrow BFU-E and CFU-E were 64.7±9.3 and 22.7±5.0/10⁵BM-MNC, respectively. Treatments with prednisolone and ALG were not effective. After thymectomy, bolus methylprednisolone failed to induce a remission.
In July, 1986, oral CsA, 200mg daily, was started. From day 27 of CsA treatment, reticulocytosis was noticeable. CsA was discontinued after 2 months. The Hb level remains stable at 14g/dl without any treatment.

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A 6-month-old infant with hemolytic anemia was transferred for further examination and treatment. Direct antiglobulin reaction by conventional antiglobulin testing was negative. However, the presence of red blood cell antibody in the serum was recognized by Polybrene test. Administration of prednisolone was effective and the diagnosis of acute (transient) autoimmune hemolytic anemia was confirmed.

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A case of B-cell chronic lymphocytic leukemia (CLL) associated with small cell lung cancer found simultaneously in a 81-year-old male is repored. To our knowledge, this is the first case reported in Japan.
He was admitted to our hospital because of further examination for his leukocytosis and generalized lymphadenopathy. Hematological studies and biopsy of lymph node revealed B-cell CLL. The chest x-ray film disclosed the huge mass lesion in the posterior segment of the right lower lobe and cancer cells (small cell type) were found in the sputum. He was treated with prednisolone and cyclophosphamide and achieved good conditions. However, 8 months after the first admission, pleural effusion and lymphangitis carcinomatosa developed and he died of pneumonia in spite of the combination chemotherapy.
CLL has been known to have a higher incidence of cancer than other types of leukemia, and immunological relationship between CLL and cancers has been emphasized, however, reports of such cases are rare in Japan.

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A 71-year-old man was admitted to our hospital because of leukocytosis in September, 1984. At the time of diagnosis of CML, all of the metaphases of bone marrow cells analyzed were Ph¹-positive without other chromosomal anomalies. He was given a new nitrosourea derivative, MCNU, in a total dose of 1050 mg, and became pancytopenic in November. Cytogenetic analysis of the bonem marrow in November showed no Ph¹ chromosome in the 20 metaphase cells. LAP activity in the peripheral blood rose to the normal level. Following MCNU-induced bone marrow hypoplasia, he maintained complete remission both hematologically and cytogenetically without therapy until June, 1985. Subsequent gradual hematological recovery was associated with an increasing percentage of Ph¹ positive cells but not with a reduction of LAP activity. Sixteen months after the karyotypic conversion all marrow cells acquired Ph¹ chromosome again, and LAP score returned to the pretreatment level 23 months after.
The patient now remains well in chronic phase of CML 30 months after the diagnosis.

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Pregnancy appears to be rare in patient with paroxysmal nocturnal hemoglobinuria (PNH). We reported two cases of successful pregnancy and delivery.
Case 1 was a 24-year-old woman with past history of acute renal failure. Laboratory findings prior to her pregnancy were as follows; Hb 10.6g/dl, RBC 327×10⁴/μl, Ht 31.7%, Ret 4.7%, WBC 5,700/μl, Plts 20.2×10⁴/μl, NAP score 11 and LDH 2,345WU. Washed packed red cells were given at the 28th and 37th week of the pregnancy. In spite of having severe toxemia, a healthy male infant was delivered by a Caesarian section.
Case 2 was a 25-year-old woman with past history of post transfusion hepatitis. Laboratory findings prior to her pregnancy were as follows: Hb 6.9/dl, RBC 224×10⁴/μl, Ht 22%, Ret 11.2% Plts 9.7×10⁴/μl, WBC 2,600/μl, NAP score 9, TBi 1.9mg/dl, LDH 5,139WU. 88 units of washed packed red cells were given during the period of pregnancy to increase Hb. At the 39th week, a healthy male infant was vaginally dilivered. She post-partum developed high fever, however, showed an improvement on antibiotic therapy.
All reported cases of pregnancy in patient with PNH were described and discussed.

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A 69-year-old Japanese male was admitted because of acute abdomen in November, 1985. Hematological examinations disclosed abnormal lymphoblasts with vacuoles resembling morphologically FAB-L3 cells in his peripheral as well as marrow blood. Those lymphoblasts had immature B cell phenotypes (J5⁺, B1⁺, OKIa-1⁺) with monoclonal nature of mu heavy and lambda light chain on their cell surface. Moreover, they had t (8; 14) (q24; q32) chromosome abnormality. Therefore, he was diagnosed to have a leukemic phase of Burkitt lymphoma or acute lymphoblastic leukemia (L3). On admission, he was severely ill with acute abdomen as well as consciousness disturbances. Although he immediately received a combination chemotherapy with vincristine, cyclophosphamide, prednisolone, and adriamycin as well as supportive therapy such as hydration, he died of renal and respiratory failure on the seventh hospital day.
Autopsy revealed no evidence of malignant lymphoma such as starry sky appearances and there was no lesion could be observed that caused acute abdomen. Burkitt cells were seen in the heart, thyroid gland, lungs, spleen, stomach, liver, gall bladder, small and large intestine, kidneys, testis, urinary bladder, prostate and some lymph nodes. Furthermore, bilateral psoas muscles were markedly infilatrated. Muscle involvement in hematological malignancies were quite rare. In 7,149 cases of hematological malignancies cited in “Annual of Pathological Autopsy Cases in Japan” from 1981 to 1983, the frequency of muscle involvement were 1.10% in non-Hodgkin's lymphoma. Acute abdomen as a initial symptom, it is quite rare as discussed.

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A case of 64-year-old female with HTLV-I associated myelopathy (HAM) is reported. She was admitted to our hospital on December 15, 1986, because of slowly progressive gait disturbance and pollakisuria that had begun 15 years before. She had prominent pyramidal tract involvement, manifesting spastic paraplegia, hyperreflexia of the extremities and pathologic reflex. On sensory examination, only vibratory sense was decreased. The white blood cell count was within normal range, however atypical lymphocytes were observed less than 1%. The cerebrospinal fluid (CSF) contained 3/3 mononuclear cells per microliter, with normal glucose and protein concentrations. These cells in blood and CSF were similar to adult T-cell leukemia/lymphoma (ATLL) cells. Both serum and CSF antibody to HTLV-I were positive by the enzyme-linked immunosorbent assay and Western blot analysis. A cranial CT scan and a myelography showed no abnormalities. Other possible causes of the myelopathy were excluded. The diagnosis of HAM proposed by Osame and colleagues was obtained.
Patients with HAM have been found mainly in ATLL-endemic area, but rarely in ATLL-non-endemic area.

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An 80 year-old man was admitted to hospital because of leukocytosis. Physical examination revealed no remarkable change. On admission, the white blood cell count was 18000/μl with a differential count of 12% band forms, 81% segmented forms and 7% others. The red blood cell count was 399×10⁴/μl. The platelet count was 7.5×10⁴/μl. NAP score was 459. Cytologic study of the bone marrow (BM) showed myeloid hyperplasia as follows: 88.8% myeloid series, 6.6% erythroid series and 4.6% others. The CFU-GM was 36/2×10⁵ BM-mononuclear cells. Cytogenetic study showed trisomy of chromosome 8 (100%) and partial deletion of the long arm of chromosome 3 (61.5%). Ph¹ chromosome was negative. Serum vitamin B₁₂ was 15830 pg/ml. Bacteriologic examinations were negative. According to these findings, a diagnosis of chronic neutrophilic leukemia was made.
The white blood cell count gradually rose to 38200/μl. About 8 months later, white cells decreased to 1500/μl, red cells to 269×10⁴/μl and platelets to 1.5×10⁴/μl without chemotherapy. On July, 6, 1985, he died of fungal pneumonia. Postmortem examination revealed hypoplastic bone marrow with some macrophages but the infiltration of neutrophils and macrophages in the liver and spleen were absent.

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A one-month-old boy with severe combined immunodeficiency (SCID) caused by adenosine deaminase (ADA) deficiency was admitted for bone marrow transplantation (BMT). Since the patient had neither sibling nor HLA identical donor, his parents were chosen as donors. Bone marrow cells from the mother were harvested and treated with monoclonal antibodies (CT-2, BA-1, -2 and -3) plus complement for depletion of both T and B lymphocytes. Although 0.5×10⁸ cells per kilogram were transfused, no remarkable reconstitution of immunologic system was observed. Two months later, the second BMT using the frozen cells from the mother was performed but was not successful. At the third and fourth BMTs, bone marrow cells from the father were treated with AET- or neuraminidase-treated SRBCs using a rosetting technique to eliminate T lymphocytes. In addition, a pre-conditioning protocol using cyclophosphamide (CY) was carried out. However, the T cell-depleted bone marrow cells were again rejected. After the third BMT, the responsiveness of the patient's peripheral lymphocytes to PHA was slightly but transiently enhanced. Basal natural killer activity also appeared about this time. This fact might be suggested as one of the reasons of continuous unsuccessful BMTs. The patient finally died of heart failure caused by a toxicity of CY. A very few cases of SCID with ADA deficiency were reported to have been corrected by HLA haploidentical BMT. Further studies regarding pre-conditioning regimens and methods of T cell-depletion are required for successful BMT in this congenital disorder.

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A case of chronic neutrophilic leukemia associated with extrahepatic portal venous obstruction is reported. A 56-year-old woman was admitted because of hematemesis and marked splenomegaly in July, 1982. Blood studies showed slight anemia and leucocytosis (33700/cmm) with 79% of mature neutrophils. The neutrophil alkaline phosphatase score was 335. Bone marrow aspiration showed a hyperplasia of myeloid elements. No evidence of Ph¹ chromosome was seen on karyotypic analysis. Esophagogastroscopy disclosed the red color sigh positive esophageal varices. To prevent bleeding from varices, the transection of the lower esophagus and splenectomy were performed in October, 1982. Operative portal venogram revealed the extrahepatic portal venous obstruction and portal venous pressure was as high as 40cmH₂O. The platelet counts were markedly elevated in the postoperative period. Bleeding tendency gradually developed. We employed busulfan, which reduced the platelet and leucocyte counts and improved these complications. She has been doing well up to March, 1987.

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A 40 year old man was admitted to the hospital due to acute myelogenous leukemia. The hemoglobin was 9.1 gr/dl and white cell count was 11,600/μl with 66% myeloblasts. The platelet count was 8.4×10⁴/μl. Bone marrow examination revealed hypercellular bone marrow with 60% myeloblasts. ECG and chest X-ray were within normal limit. Remission induction therapy was treated with BH-AC. DMP (BH-AC 250 mg, DNR 60 mg, 6MP 70 mg, PSR 40 mg). As complete remission was not obtained by one course of BH-AC. DMP therapy, the second course of the BH-AC. DMP therapy was started. Three minutes after the slow infusion of daunorubicin (60 mg) on the first day of the second course, the patient suddenly lost consciousness and underwent a cardiac arrest. Resuscitation attempts were succesful and unconsciousness was recovered 15 minutes after the shock. Transient I° A-V block was recognized by ECG, but the values of GOT, LDH and CPK were not changed. Cardioechography and Holter ECG after the shock revealed no change. This accident occured by acute cardiac dysfunction due to daunorabicin administration. The importance of careful observation of the patient receiving daunorubicin was stressed, because the acute cardiac dysfunction was possibly to be happened.

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A 69-year-old female was admitted with high fever abdominal pain. Laboratory findings showed high serum LDH (2930W.U) and thrombocytopenia (5.2×10⁴/μl ). CT revealed hepatosplenomegaly, ascites and mild pleural effusions. Bone marrow aspiration showed normocellularity with 23.4% immature atypical cells showing various range of maturation and cytophagocytosis by mature histiocytes (1.2%). Cytogenetic study delineated a clone with 48, XX, 1q+, 1p-, 7q+, 9p+, +12, 13q-, -14, 17p+, 18p+, 19q+, +mar, +mar. This patient progressively deteriolated with icterus, bleeding tendency and ventricular arrythmias and suddenly died of fatal arrythmia. At the terminal stage, many immature cells appeared in the peripheral blood, which provided the opportunity of studying by electron microscopy, by cell membrane marker classification and also by culture of malignant cells. These findings suggested the histiomonocytic origin of the abnormal cells. The diagnosis of malignant histiocytosis was based on clinical manifestation, morphologic features, immunocytochemical studies and chromosomal abnormalities.

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